Background: Thrombosis in light chain amyloidosis (LCA) occurs in the context of multiple organ dysfunction and inflammation. Conventional coagulation tests (screening) may not sufficiently capture the procoagulant substrate in the inflammatory/therapeutic dynamics. Methods: A total of 61 consecutive patients with LCA were prospectively included in the study. Clinical data, including organ involvement, time of diagnosis, treatment phase, DOAC exposure and thrombosis history were systematically recorded and subjected to screening. Specialized hemostasis tests such as APTT/PT, fibrinogen, D-dimer, TEG and TGT were performed and conventional times were analyzed in the subgroup without DOAC. Results: The prevalence of documented thrombosis was 32.8%, and thrombosis status was associated with TEG positivity and more strongly with TGT positivity. Hypercoagulability was identified in 50.8% by TEG and 41.0% by TGT, regardless of whether APTT/PT were within the reference values. APTT/PT did not predict thrombosis recurrence (p > 0.05), which was predicted by TEG (p = 0.0027) and TGT (p = 0.0006). An inflammation/fibrin turnover panel (CRP, fibrinogen, D-dimer) predicted TEG positivity (p < 0.0001), but not TGT, and was correlated with assessment at diagnosis, daratumumab-based therapy, and cardiac involvement. Conclusions: Global tests (TEG/TGT) promptly correlate with thrombosis recurrence in our cohort and provide crucial information in addition to clotting times for thrombotic phenotyping. Inflammation can influence TEG, so the decision to recommend the tests and the timing of their performance should be adapted to the clinical, biological, and therapeutic context.

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy requiring prompt diagnosis and treatment. This retrospective single-center study analyzed 31 adult patients diagnosed between 2013 and 2024 (PLASMIC score ≥ 5, ADAMTS13 activity < 10%), aiming to characterize their clinical profiles and assess the impact of caplacizumab. Methods: Baseline laboratory parameters (platelet count, LDH, creatinine, hemoglobin, number of plasmapheresis sessions, number of hospitalization days, number of days in intensive care, and days required to recover platelet count) were included in statistical analysis to predict diverse outcomes, such as respiratory distress, infection, major neurological manifestations, gastrointestinal involvement, or refractoriness/exacerbation. Sixteen patients underwent treatment with caplacizumab in addition to plasmapheresis (PEX) and corticosteroids, while the remainder received PEX and corticosteroids alone. Results: Our predictive models proved noteworthy, providing results with ROC values ranging from 0.80 to 0.90 (p < 0.01). Caplacizumab was associated with faster platelet recovery (4 days vs. 7 days), fewer PEX sessions, shorter hospital stays, and a significantly lower incidence of refractoriness or exacerbation (p < 0.05). Inter-group analysis confirmed a significant reduction of overall resource use (p < 0.05). Conclusions: Early caplacizumab use improved outcomes and optimized resource utilization. This real-world study suggests that routinely available laboratory markers at presentation can help predict outcomes and guide early clinical decisions in centers without rapid ADAMTS13 testing.